Abstract:

Wen Sijian*, Hu Bin, Yang Li, Song Hao, Xiong Jingshu, Zhang Wei, Xu Xiulian, Jiang Yiqun, Chen Hao, Zeng Xuesi, Sun Jianfang. *Department of Pathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China Corresponding authors: Chen Hao, Email: ch76ch@163.com; Sun Jianfang, Email: Sunjf57@163.com 【Abstract】 Objective To investigate the diagnosis and differential diagnosis of dermatofibrosarcoma protuberans （DFSP）. Methods Totally, 50 patients with DFSP visiting the Institute of Dermatology, Chinese Academy of Medical Sciences from 1998 to 2014 were enrolled. The clinical manifestations, histopathological and immunohistochemical features, treatment and prognosis of DFSP were retrospectively reviewed. Results The average age at onset of DFSP was （29.5 ± 15.9） years in the 50 patients, with a mean disease duration of 9.57 years. Skin lesions most frequently occurred on the trunk （n = 33, 66.0%）, followed by the extremities, head and neck. DFSP was characterized by atrophic patches or plaques in 13 cases （26.0%）, multiple nodules varying in size and arising on atrophic plaques or patches in 30 cases （60.0%）, single or multiple nodules arising on normal skin in 7 cases （14.0%）. Histologically, the tumor consisted of uniform infiltrative spindle cells arranged in a storiform or cartwheel pattern. In addition, the tumor cells expressed CD34 and vimentin. Twenty patients experienced recurrence at the primary site after resection of skin lesions with a recurrence rate of 43.5%. No distant metastasis or death occurred in these patients. Conclusions DFSP usually has various skin manifestations, is easily misdiagnosed, and can be confirmed based on histopathological and immunohistochemical findings. Local recurrence of DFSP is common, and may occur for many times after surgical excision, but lymphatic and distant metastases are rare.